| Early bone marrow transplantation in an infant with Wiskott-Aldrich syndrome. | |
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MedLine Citation:
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PMID: 1793157 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The Wiskott-Aldrich Syndrome (WAS) is a rare X-linked immunohematological disorder characterized by eczema, profound thrombocytopenia, and progressive immunodeficiency. Severe hemorrhage, overwhelming sepsis, or lymphoreticular malignancy usually cause death in childhood. Recently, bone marrow transplantation (BMT) has been curative in some well-established cases, but there is no general agreement about the place of BMT in infants with WAS before the development of significant immunological abnormalities. We describe the successful use of early histocompatible BMT in a 10-month-old infant in whom WAS was diagnosed on the basis of eczema, thrombocytopenia, small platelets, and raised serum immunoglobulin A (Ig) and IgE, but before the development of immunodeficiency as evidenced clinically by recurrent infections, or immunologically by low serum IgM or consistently abnormal lymphocyte responses to mitogens. After an unstable period for several weeks posttransplantation when he developed marked hepatomegaly and severe interstitial pneumonitis, he made a good recovery. His eczema and thrombocytopenia resolved and he has shown no clinical or laboratory evidence of immunodeficiency. It is now over 2 years since his BMT. Because of the poor prognosis of WAS, where a histocompatible donor is available, BMT at the earliest opportunity, despite the inherent risks of such a procedure, may be the best option for an infant with WAS. |
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Authors:
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L J Beard; I R Toogood; C C Pearson; A Ferrante |
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Publication Detail:
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Type: Case Reports; Journal Article |
Journal Detail:
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Title: The American journal of pediatric hematology/oncology Volume: 13 ISSN: 0192-8562 ISO Abbreviation: Am J Pediatr Hematol Oncol Publication Date: 1991 |
Date Detail:
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Created Date: 1992-04-02 Completed Date: 1992-04-02 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 7908071 Medline TA: Am J Pediatr Hematol Oncol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 310-4 Citation Subset: IM |
Affiliation:
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Department of Immunology, Adelaide Children's Hospital, South Australia. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Age Factors Bone Marrow Transplantation* / adverse effects Follow-Up Studies Humans Immunocompromised Host Immunoglobulins / analysis Infant Leukocyte Count Male Platelet Count Pulmonary Fibrosis / etiology Wiskott-Aldrich Syndrome / blood, immunology, surgery* |
| Chemical | |
Reg. No./Substance:
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0/Immunoglobulins |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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